A Role of brachytherapy in bilateral Wilms tumors: A long-term follow-up of three highly selected cases and literature review.

PURPOSE: To describe experience with partial nephrectomy combined with brachytherapy as part of the local management of bilateral Wilms tumor (WT) including a review of the available literature. RESULTS (METHODS AND CASE DESCRIPTION): Between 2011 and 2014, three highly selected patients (age nine months, 16 months, and 4 years) with bilateral WT (two synchronous and one metachronous) underwent enucleation and perioperative brachytherapy to the tumor bed. With a minimum follow-up of 5 years, all three patients are in continuous complete remission with preserved kidney function. CONCLUSIONS: Although nephron sparing surgery aiming at tumor free-margins remains the gold standard for bilateral WT, tumor enucleation followed by brachytherapy may be considered in carefully selected patients at high risk for end-stage kidney failure. Given the rarity and complexity of the procedure, concentration of care of such patients is mandatory.

The SIOP-Renal Tumour Study Group consensus statement on flank target volume delineation for highly conformal radiotherapy.

For decades, radiotherapy with two opposing photon beams has been the standard technique used to cover the flank target volume in paediatric patients with renal tumours. Nowadays, many institutes are implementing advanced radiotherapy techniques that spare healthy tissue. To decrease the radiotherapy dose to healthy structures while preserving oncological efficacy, the conventional approach of flank irradiation has been adapted into a guideline for highly conformal flank target-volume delineation by paediatric radiation oncologists and representatives of the International Society of Paediatric Oncology's Renal Tumour Study Group (SIOP-RTSG) board during four live international consensus meetings. The consensus was refined by delineation exercises and videoconferences by ten collaborating paediatric radiation oncologists. The final guideline includes eight chronological steps to generate the tumour bed and clinical, internal, and planning target volumes, and it describes the optional use of surgical clips to optimise treatment planning. This guideline will be added into the radiotherapy guideline of the UMBRELLA SIOP-RTSG protocol for paediatric renal tumours to improve international consistency of highly conformal flank target-volume delineation.

Lung function of infants with congenital lung lesions in the first year of life.

BACKGROUND: Several studies have evaluated short-term neonatal outcome in infants with congenital lung lesions (CLL) but clinical course and lung function in the longer term have not yet been documented. We hypothesized that clinical course and lung function would be negatively affected by surgical resection. OBJECTIVE: To evaluate respiratory symptoms and lung function longitudinally in the first year of life in infants with CLL, and to analyse differences herein between infants managed by observation only and infants whose affected lung parts were resected. METHODS: We evaluated respiratory symptoms and lung function at 6 and 12 months in 30 patients with CLL. Functional residual capacity (FRC(p)) and maximal expiratory flow at functional residual capacity (V'(max)FRC) were measured with body plethysmography. SD scores were calculated for V'(max)FRC. RESULTS: Prevalence of respiratory symptoms did not differ between the groups. Mean FRC(p) (95% CI) was 25.3 (23.3-27.3) in the group managed by observation versus 27.3 (25.1-29.6) in the group managed by surgery (p = 0.149). Mean (95% CI) SDS V'(max)FRC was -1.45 (-1.84 to -1.06) versus -1.41 (-1.90 to -0.91) (p = 0.892). Lung function did not change significantly over the 6-month period. CONCLUSION: Surgical resection did not seem to have negatively affected the clinical course and lung function. We recommend pulmonary follow-up of all CLL patients into adulthood to further identify any long-term effects of CLL and observation or surgery.

Congenital diaphragmatic hernia: to repair on or off extracorporeal membrane oxygenation?

BACKGROUND: Congenital diaphragmatic hernia (CDH) can be repaired on or off extracorporeal membrane oxygenation (ECMO). In many centers, operating off ECMO is advocated to prevent bleeding complications. We aimed to compare surgery-related bleeding complications between repair on or off ECMO. METHODS: All patients with CDH repair and ECMO treatment between January 1, 1995, and May 31, 2008, were retrospectively reviewed. Tranexamic acid was routinely given to all patients repaired on ECMO for 24 hours perioperatively after 2003. Extra-fluid expansion, transfusion, or relaparotomy caused by postoperative bleeding were scored as surgery-related bleeding complications and were related to the Extracorporeal Life Support Organization (ELSO) registry. We used χ(2) test and t test for statistics. RESULTS: Demographic data and surgery-related bleeding complications in the on-ECMO group were not significantly different compared with the off-ECMO group (P = .331) in our institute. In contrast, more surgery-related bleeding complications were reported by ELSO in their on-ECMO group (P < .0001). CONCLUSION: In contrast to the data from the ELSO registry, we did not observe significantly more surgery-related bleeding complications after CDH repair on ECMO. Using a specific perioperative hemostatic treatment enabled us to perform CDH repair on ECMO with a low frequency of bleeding complications, thereby taking advantage of having the physiologic benefits of ECMO available perioperatively.